Usual interstitial pneumonia is the most common idiopathic interstitial pneumonia31 and carries the worst prognosis.30 UIP is the radiologic and pathologic pattern observed in patients with IPF,45 but UIP-like patterns are also caused by secondary conditions such as collagen vascular disease, chronic hypersensitivity pneumonitis, asbestosis, or drug toxicity,32,33 highlighting the importance of identifying any possible causes of lung disease prior to diagnosing idiopathic UIP.45

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Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Reference

30. Travis WD, Matsui K, Moss J, Ferrans VJ. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol. 2000;24:19-33.

31. Spagnolo P, Tonelli R, Cocconcelli E, Stefani A, Richeldi L. Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges. Multidiscip Respir Med. 2012;7:42.

32. Takemura T, Akashi T, Kamiya H, et al. Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia. Histopathology. 2012;61:1026-1035.

33. Glazer CS, Newman LS. Occupational interstitial lung disease. Clin Chest Med. 2004;25:467-478.

45. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68.